Fibrocystic disease of the pancreas in the newborn.

Fibrocystic disease of the pancreas in the newborn infant is usually recognized by the development of intestinal obstruction as a result of meconium ileus (Andersen, 1938). According to Bodian (1952), a certain number of infants affected by fibrocystic disease develop severe intestinal lesions during intrauterine life. The abnormal consistence of the mucus produced in the alimentary tract causes inspissation of meconium and leads to intestinal obstruction, perforation or even atresia of the bowel. In the older infant the disease is more likely to take the form of a severe nutritional disorder which is liable to be complicated by respiratory infection. In a number of patients the respiratory disorder may dominate the clinical picture (Farber, 1944). Intestinal obstruction is rare in this group of patients, but it has been reported by Levy (1951) and by Fisher (1954) as a late complication of fibrocystic disease. On the other hand, a congenital disorder such as fibrocystic disease of the pancreas is liable to be undetected in the neonatal period unless it appears as meconium ileus. Bodian (1952) states that in his series no children died in the first days of life with fibrocystic disease other than those with meconium ileus. He was thus unable to compare the distribution of intestinal lesions in infants of this age group with and without meconium ileus. In this laboratory we have examined sections of the pancreas obtained from a consecutive series of 500 necropsies on newborn infants without finding a single example of fibrocystic disease. Recently, however, a case was encountered of an infant who died from anoxia during delivery. At post-mortem examination severe fibrocystic disease of the pancreas was found and lesions were also visible in the liver. There was no evidence of intestinal obstruction and the large bowel was full of meconium. This appears to be the first recorded example of fibrocystic disease of the pancreas without an intestinal lesion in a newborn infant. Case Report